Abnormalities in polyprenol metabolism appear to be the underlying cause in Refsums disease and in ceroid-lipofuscinosis that are clinically characterized by retinitis pigmentosa. In Refsums disease, phytanic acid and in ceroid-lipofuscinosis, dolichol, are excreted in large amounts in the urine. The objective of the proposed study is to determine whether there are any excessive excretions of polyprenol related compounds in the urine of patients with non-neurological retinitis pigmentosa. Urinary lipids from various forms of the disease (autosomal recessive, autosomal dominant and x-linked) will be analyzed for phytanic acid, unsaturated phytanic acid, phytol and dolichol by a combination of thin-layer chromatography, high pressure liquid chromatography and high resolution capillary gas-liquid chromatography. Preliminary data suggest a four to six times the normal level of dolichol in two patients with retinitis pigmentosa. Knowledge about the storage material in retinitis pigmentosa will enable to design experiments in the study of the basic biochemical defect in this disease. Further, polyprenol levels inurine may provide a specific classification of this heterogenous group of diseases and possibly provide a diagnostic tool.